Papillary Fibroelastoma of the Mitral Valve with ... - Wiley Online Library

0 downloads 0 Views 704KB Size Report
We present the case of a papillary fibroelastoma of the mitral valve with systemic embolization in a young man. (ECHOCARDIOGWHY, Volume 17, February ...
Papillary Fibroelastoma of the Mitral Valve with Systemic Embolization HAYRETTIN KAFWEREN, M.D.," T. FIKRET ILGENLI, M.D.," TURGAY CELIK, M.D.," SALIH DEVECI, M.D.,? ERKAN KURALAY, M.D.,$ CEM BARCIN, M.D.," MEHMET UZUN, M.D.," CELAL GENC, M.D.," and ERTAN DEMIRTAS, M.D., F.A.C.C." Departments of "Cardiology, ?Pathology, and $Cardiovascular Surgery, Gulhane Military Medical Academy, Ankara, Turkey

Primary tumors of the heart are rare disorders. In autopsy studies, their incidence was reported to be 0.01-0.5%. We present the case of a papillary fibroelastoma of the mitral valve with systemic embolization in a young man. (ECHOCARDIOGWHY,Volume 17, February 2000) papillary fibroelastoma, mitral valve, echocardiography We present the case of a papillary fibroelastoma of the mitral valve with systemic embolization in a young man. Case Report

A 21-year-old man who was previously asymptomatic was admitted to our hospital neurology clinic with the complaint of weakness of his left extremities. His physical examination was normal except for weakness of his left extremity muscles. He was hospitalized, and a computed tomography scan of the head was performed, which revealed a n area of infarct in the right portion of the brain. He was referred to the cardiology department. Transthoracic echocardiography was performed to search for a source of systemic embolization echocardiography (Figs. 1and 2). A mobile mass with a diameter of 1.5 cm was revealed on the atrial aspect of the anterior mitral leaflet. On the apical four-chamber view, the mass appeared to have the shape of a ball. Transesophageal echocardiography was

Address for correspondence and reprint requests to: Hayrettin Karaeren, M.D., Department of Cardiology, GATA Etlik, Ankara, Turkey. Fax: 0090-312-3234923.

Vol. 17,No.2,2000

performed and clearly demonstrated the lesion (Fig. 3). The patient underwent surgery on an urgent basis. The lesion was excised, and the anterior mitral leaflet was reconstructed by cardiac surgeons. Macroscopically, the mass was 1.5 cm in diameter and shiny white, with a thick core surrounded by papillary foldings with a characteristic flower- or sea anemone-like appearance. Histopathologically, the tumor had typical features of papillary fibroelastoma (Fig. 4). Papillary fronds of tumor were narrow and elongated and had complex branching. The papillae were avascularized and covered by a single layer of endothelial cells. The matrix consisted of hyalinized fibrous tissue with occasional spindle cells resembling smooth muscle cells or fibroblasts.

Discussion Primary tumors of the heart are rare disorders.' In autopsy studies, their incidence was reported to be 0.01%-0.5%. Approximately 75%-80% of these tumors are benign and can be easily treated surgically . Papillary fibroelastomas account for 7%-8% of cardiac tumors. They are the most common

ECHOCARDIOGRAPHY: A Jrnl. of CV Ultrasound & Allied Tech.

165

KARAEREN, ET AL.

Figure 1. Transthorasic echocardiogram. Parasternal long-axis view shows a mass beneath the anterior leaflet of the mitral valve.

Figure 3. Transesophageal echocardiogram clearly showing the mass at the beginning of diastole. The aortic valves are not yet closed.

tumors of cardiac valves. Usually, they are discovered post mortem, with an autopsy incidence of 0.002%-0.33%, but their diagnosis is now easy with the use of two-dimensional echocardiog~aphy.~-* Yee et al.5 described echocardiographic features in their recent review. These lesions are pedunculated finger-like mobile masses on one or more of the cardiac valves. Their size may vary from a few milli-

meters to a few centimeters. They are attached to endocardial sites of intracardiac native valves by small stalks and are located on the mid-portion or body of the valve away from the contact surface. These lesions have a more defined acoustic interface than vegetation. They typically are located on the atrial surface of the atrioventricular valves (as in our patient) and on the ventricular surface of the semilunar valves. They can arise from any endocardial surface, such as interatrial septum.6 They are

Figure 2. Transthorasic echocardiogram. Apical four-chamber view shows a ball-like mass at the atrial part of anterior leaflet of the mitral valve.

Figure 4. Avascular hyalinized fibrous fronds covering endothelial cells in the papillary fibroelastoma (hematoxylin and eosin, magnification 25 X).

166

ECHOCARDIOGRAPW: A Jrnl. of CV Ultrasound & Allied Tech.

Vol. 17, No. 2, 2000

PAPILLARY FIBROELASTOMA WITH EMBOLIZATION

usually found in patients > 60 years old, but they have been reported in patients 23-86 years old. Our patient is the youngest patient with the diagnosis of papillary fibroelastoma. Many of the tumors are clinically silent, but papillary fibroelastoma has been well documented as a possible cause of ischemic stroke.3,7,8The diagnosis of these tumors as the cause of ischemic stroke should be made only through exclusion. Mitral valve tumors are more likely than aortic valve tumors to produce serious neurological symptoms or sudden death.5.6 Yee at al.5 reported 15 patients with papillary fibroelastoma and found that 35% of patients (5 of 15) were diagnosed with ischemic stroke from left-sided papillary fibroelastoma through exclusion. Our patient was evaluated for other possible causes of stroke, but wedid not find carotid stenosis, thrombi or smoke in the heart chambers, pedunculated atherosclerotic excrescence in the arcus, or ascending aorta or coagulation abnormalities. We therefore consider the cause to be papillary fibroelastoma. These tumors have also been associated with angina, conduction disturbances, and valvular dysfunction.lF9.10 These types of lesions have multiple papillary fronds that resemble a sea anemone or flower. They may be single or multiple and can be up to 5 cm in diameter.7 These lesions can form a nidus for platelet and fibrin aggregation and can lead to systemic or neurological emboli. The management of these tumors is controversial. Some authors suggest that all tumors be resected, even in asymptomatic patients.11 Yee et al.5 suggest the surgical removal of all symptomatic papillary fibroelastomas if patient will undergo open heart surgery for another reason. Right-sided tumors are rarely symptomatic, and surgery is not recommended for asymptomatic lesions. If a left-sided tumor is asymptomatic, there are three options: surgery, anticoagulation, or regular follow-up. In conclusion, papillary fibroelastoma of the

Vol. 17, No. 2,2000

heart must always be kept in mind in patients who have had a stroke. Acknowledgment: The authors thank Murat Salin for typing the manuscript.

References 1. Salcedo EE, Cohen GI, White RD et al: Cardiac tumors: Diagnosis and management. Curr Probl Cardiol 1992;17:73. 2. LiMandri G, Homma S, Di Tullio MR, et al: Detection of multiple papillary fibroelastomas of the tricuspid valve by transesophageal echocardiography. J A m SOCEchocardiogr 1994;7: 315. 3. Shahian DW, Labib SB, Chang G Cardiac papillary fibroelastoma. Ann Thorac Surg 1995;59: 538. 4. Shub C, Tajik AJ, Seward JB, et al: Cardiac papillary fibroelastomas: Two dimensional echocardiographic recognition. Mayo Clin Proc 1981;56:629-633. 5. Yee HC, Nwosu JE, Lii AD, et al: Echocardiographic features of papillary fibroelastoma and their consequences and management. Am J Cardiol 1997 80:811-814. 6. Edwards FH, Hale D, Cohen A, et al: Primary cardiac valve tumors. Ann Thorac Surg 1991; 52:1127-1133. 7. Burke A, Virmani R Tumors of the heart and great vessels. In: Atlas of Tumor Pathology, 3rd series. Armed Forces Institute of Pathology, Washington, DC, 1996,pp. 47-54. 8. Colucci V, Alberti A, Bonacina E, et al: Papillary fibroelastoma of the mitral valve: A rare cause of embolic events. Tex Heart Inst J 1995; 22:327-331. 9. Pomerance A Papillary "tumors" of the heart valves. J Pathol Bacteriol 1981;87:135. 10. Kasarkis EJ, O'Connor W, Earle G: Embolic stroke from cardiac papillary fibroelastomas. Stroke 1988;19:1171-1173. 11. Allen KB, Goldin M, Mitra R: Transaortic video-assisted excision of a left ventricular papillary fibroelastoma. J Thorac Cardiovasc Surg 1996;112:199-201.

ECHOCARDIOGRAPW: A Jml. of CV Ultrasound & Allied Tech.

167